Developmental Disability and Ageing

Developmental

Disability and Ageing

Developmental Disability and Ageing

Edited by

Gregory O’Brien

and Lewis Rosenbloom

2009

Mac Keith Press

Distributed by Wiley-Blackwell

© 2009 Mac Keith Press

6 Market Road, London N7 9PW, UK

Editor: Hilary M. Hart

Managing Director, Mac Keith Press: Caroline Black Project Manager: Sarah Pearsall

The views and opinions expressed herein are those of the authors and do not necessarily represent those of the publisher

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission of the publisher

First published in this edition 2009

British Library Cataloguing-in-Publication data

A catalogue record for this book is available from the British Library

ISBN: 978–1–898683–61–2

Typeset by Keystroke, 28 High Street, Tettenhall, Wolverhampton Printed by The Lavenham Press Ltd, Water Street, Lavenham, Suffolk Mac Keith Press is supported by Scope

To Michael Pountney, who never gave up

Contents

List of Contributors

How to Use this Handbook

1  Overview: Development Disability and Ageing

Gregory O’Brien and Lewis Rosenbloom

2  Dementia in Developmental Disability

Mohammed Nagdee and Gregory O’Brien

3  Ageing in Down Syndrome

Tom Berney

4  Cerebral Palsy and Ageing

Gregory O’Brien, Alf Bass and Lewis Rosenbloom

5  Ageing in Other Syndromes

Marc Woodbury-Smith

6  Drug Treatment for Common Problems among Elderly People with Developmental Disabilities (including Dementia)

Christopher Ince

7  Living with Ageing in Developmental Disability

Claire Middleton and Gregory O’Brien

References

Index

Contributors

Alf Bass, Consultant Orthopaedic Surgeon, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK

Tom Berney, Honorary Consultant, Northumberland, Tyne and Wear NHS Trust, UK

Christopher Ince, Consultant in Forensic and Forensic Learning Disability Psychiatry, Northgate Hospital, Morpeth, Northumberland, UK

Claire Middleton, Assistant Psychologist, North Wales NHS Trust, UK

Mohammed Nagdee, Principal Consultant Psychiatrist, Fort England Hospital, Grahamstown, South Africa

Gregory O’Brien, Professor in Developmental Psychiatry, Northgate Hospital, Morpeth, Northumberland, UK

Lewis Rosenbloom, Honorary Consultant Paediatric Neurologist, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK

Marc Woodbury-Smith, Assistant Professor, Offord Centre for Child Studies, Hamilton, Ontario, Canada

How to Use this Handbook

This handbook is aimed at clinicians and others who are engaged in caring for ageing adults with developmental disabilities. The text is intended to inform understanding, to promote assessment, to assist in care planning, and especially to improve everyday living for this needy but sadly often neglected group of vulnerable individuals.

The text is not an academic tome. While the statements made are all based on sound evidence, no attempt is made to comprehensively review all the research evidence. The approach taken has been to cite crucial evidence – whether long-established, or new – and to indicate sources of further reading, while focusing on important insights which are likely to be valuable to the clinician interested in the care of the individuals on whose behalf the book has been prepared.

Chapter 1 is a brief general overview of the area. Chapter 2 is a detailed consideration of dementia in the context of developmental disability, including cause, diagnosis, assessment and natural history, with case examples. Chapters 3 and 4 concentrate on two of the most high-profile of all the major groups of developmental disabilities, with their own unique patterns of ageing, Down syndrome and cerebral palsy. Chapter 5 reviews other, less common causal syndromes, and their characteristics with ageing. Chapter 6 is a detailed guide to drug treatment issues in this group. Finally, Chapter 7 considers wider issues of psychosocial intervention and life planning for the ageing individual with developmental disability.

It is likely that the interested clinician will find that the book is merely a pointer to further reading and consideration of the health and social care needs of elderly people with developmental disability. If so, then one of the main aims of the book will have been achieved.

Gregory O’Brien and Lewis Rosenbloom

Chapter 1

Overview: Developmental Disability and Ageing

Gregory O’Brien and Lewis Rosenbloom

Introduction

Children get the service, but adults lead the life.

This sentiment has long been a concern among those who advocate for better care of people with developmental disabilities. To this statement might now be added the concern that:

… the older they get, the worse it can be for them.

Previously, the notion of having a population of older adults with serious developmental disabilities was not widely considered. Most efforts in care, treatment, service development and planning were therefore focused on children. Now, thanks to major recent innovations in health and social care, there is a steadily growing population of elderly adults with developmental disabilities. The health and general care needs of this group, and how best these should be met, are the focus of this text. Beginning with the overview and background review of the present chapter, and moving on to, first, consideration of the major challenges of dementia among this group, followed by detailed exploration of major causes of disability and the special problems older age brings – with chapters on Down syndrome, cerebral palsy and other congenital syndromes of disability – and finally reviews of the special considerations that need to be made in prescribing medication and planning care for the older adult with developmental disability; this brief handbook aims to bring practical insights to physicians, other professionals and carers dedicated to improving the lives of this deserving group.

Life expectancy: general population and developmental disability

People are living longer. This much has been known for a long time, and the trend is set to continue for a long time to come. The old idea of life expectancy being ‘three score years and ten’ seems a distant memory, as normal life expectancy in the general population increases steadily. Figs 1.1 and 1.2 show the projected life expectancy for the UK population: both measures used in official government data are shown here – period expectancy and cohort expectancy. Period life expectancy is the more often quoted figure – this shows that, for 2004, the UK general population life expectancy for men was 84, and for women 87 years. This rising life expectancy is largely due to advances in nutrition, and in medical and care services.

The corresponding rise in the life expectancy of adults with developmental disabilities has already had far-reaching consequences (Janicki et al 1998). In the UK, a recent government White Paper (in the UK, a White Paper is a statement of government policy – the data cited and reviewed therein are the officially accepted figures, on which services are planned) indicates that there are over 200,000 people over the age of 60 years with intellectual disabilities. Fig. 1.3 illustrates the proportion of the general population that this represents (Department of Health 2001). The same White Paper estimates that the population of people with intellectual disability will increase by 1 per cent per year for at least the next 15 years, this increase being mainly attributable to increased longevity among adults with intellectual disabilities. In the USA, the former American Association for Mental Retardation (re-named the ‘American Association for Intellectual Disability’ from 2006) estimates that there are between 600,000 and 1.6 million adults over the age of 60 years with intellectual disabilities and other similarly disabling developmental disabilities. The most recent USA estimate for the average life expectancy of adults with intellectual disability is 66 years and rising (Fisher and Kettl 2005).

Figure 1.1 UK life expectancy: women – period and cohort expectancies

Figure 1.2 UK life expectancy: men – period and cohort expectancies

Note: These figures for life expectancy are taken from the UK Government Actuary’s Department, 2004. Period life expectancy is worked out using age-specific mortality rate for a given year, making no allowance for any later actual or projected changes in mortality. Period life expectancy is the more usually quoted and used figure for populations. Cohort life expectancy allows for known or projected changes in mortality in later years.

Figure 1.3 Estimated numbers of people with intellectual disabilities (ID) in the UK, 1999

Source: Department of Health 2001.

These figures need to be placed in context when we move on to consider older adults with developmental disabilities. For, just as these increases in life expectancy among people with significant developmental disabilities are a reflection of improved health and other services, so it follows that it is only through such continued improvements that these increases will continue to be seen. Also, it is well recognized by most people that the concept of average life expectancy in the general population (as outlined in Figs 1.1 and 1.2) is very much an overall arithmetical average, behind which lies great individual variation, due to differing health conditions, nutrition, etc. Such individual variation is much greater among people with developmental disabilities, for a number of notable reasons. First, many of the individual causes of developmental disability feature other health-threatening and compromising conditions, which correspondingly impact upon life expectancy, especially in certain genetic syndromes of disability and other aetiologies. These matters are explored in detail in Chapters 3, 4 and 5, in respect of Down syndrome, cerebral palsy, and other genetic conditions, respectively. Second, there is a strong association between more severe and profound intellectual disability, and a shorter life expectancy. Similarly, the presence of multiple and complex disabilities has the effect of shortening life expectancy. Finally, however, the best available evidence is that, not only can life expectancy increase among people with severe and complex disabilities, but it should do so – because there is a high avoidable mortality among this group. The most recent estimates are that this avoidable mortality is of the order of 10 times. This was highlighted recently in the UK by the Disability Rights Commission, which has carried out a detailed and authoritative inquiry into inequalities in health care among people with disabilities (Disability Rights Commission 2006).

Deteriorating physical health and health care planning

Increased longevity among people with developmental disabilities is increasingly contributing to a population of elderly individuals which suffers from states of greater physical deterioration and dependency. The challenges posed here, in terms of physical debility and locomotor problems, are the subject matter of Chapter 4, which focuses on cerebral palsy, and especially on how physical disability progresses among older adults with cerebral palsy, and how these challenges can be met.

One of the most important principles to bear in mind in planning health care for older adults with developmental disabilities is: get the balance right between expecting deteriorating functioning, and accepting it. Take, for example, the older person with Down syndrome (see Chapter 3). All involved in the care of the individual must be on guard to recognize that premature ageing is common – but not to accept all apparent signs of such as premature ageing, necessarily. Hypothyroidism in particular must be detected, and treated, as must depression and other problems. Further, where dementia is diagnosed, there is immense scope for intervention, through medication (Chapter 6) and careful attention to daily life planning and other interventions (Chapter 7). A major element of this approach lies in educating carers about the features, symptoms and natural histories of the major age-related conditions – especially dementia (Chapters 2 and 7) – so that they can be well-informed regarding both what lies ahead for the people whom they serve, and, equally, what can be done to maximize the person’s health and functioning, especially in the face of organic deterioration (Nochajski 2000). Commenting on this matter, Hogg and Lambe (1998) have emphasized the value and advantages of careful assessment of health problems and disability among older people with developmental disabilities, in order to identify remediable and reversible health problems and also to plan for other problems in which progressive loss of functioning is inevitable.

In addition to the special attention which needs to be paid to the specific health problems of the different causes of developmental disability (see Chapters 3, 4 and 5), it should be remembered that the same common conditions which are responsible for the bulk of morbidity and mortality in later life in the general population are also at least as common among older people with developmental disabilities (Holland 2000). Heart disease, hypertension and other circulatory problems; cancer of the major internal organs; major endocrine problems such as diabetes, with all its complications; rheumatoid and osteo-arthritis and other major skeletal problems: all of these common serious conditions are common among this population, particularly among those who share the same predisposing factors such as diet, smoking and, in certain conditions, family history. However, many of these individuals are far less likely – whether through ability or opportunity – to give voice to their symptoms. For this reason, it is even more important that targeted, pro-active health screening should be carried out among this population, to identify and treat emerging conditions. Such targeted screening needs to be focused also on gender-specific problems, such as breast cancer and testicular cancer, which can be easily missed if regular screening examinations are not carried out (Davidson et al 2003).

Cognitive decline and dementia

One of the greatest challenges facing all who care for people with developmental disabilities is a direct and inevitable consequence of the changes in the age structure of the population: the increasing burden of cognitive decline and dementia in developmental disability. This is the focus of Chapter 2, which reviews clinical presentation, differential diagnosis, investigations and natural history of dementia in developmental disability; treatment and care planning are the focus of Chapters 6 and 7.

The challenge of dementia among people with developmental disability is at its most familiar in the most common cause of intellectual disability, Down syndrome, in which dementia is particularly common, and indeed often occurs early in adult life (see Chapter 3). Carers need to be alert to the changes in self-care and functioning which are typical of dementia as it presents in this population, and to be aware of how this varies with pre-morbid intellectual level, as well as how to adapt towards the needs of this special group the interventions which have been developed for dementia in the general population. Also, there is growing awareness that, as in the general population, normal age-related cognitive decline among older adults with intellectual disabilities must be differentiated from dementia (Holland 2000). In other words, just as in the general population, older people with developmental disability may become mildly forgetful, while not necessarily developing dementia. However, there is as yet little detailed description available of normal age-related cognitive decline in intellectual disability. One key to this distinction is the growing literature on dementia among this population (e.g. Cooper 1997a).

Mental health

Diagnosis of mental health problems among all people with developmental disability poses challenges. All too often, symptoms of depression, anxiety or even of more serious mental illness are ascribed or disregarded as features or ‘understandable’ consequences of having serious disability. This ‘diagnostic overshadowing’ has been shown to be even more likely to occur among elderly adults with intellectual disability. Nevertheless, it is clear that older